G315(P) Treatment of anticardiolipin antibody mediated chorea in an adolescent male

Abstract

BackgroundAnti-phospholipid antibody associated chorea is a rare cause of this movement disorder, with a higher incidence in females and children. Other causes include Wilson’s disease, Sydenham’s chorea and ataxia-telangiectasia. Aetiology is uncertain, but may involve anti-phospholipid mediated dysfunction of the basal ganglia. We report an unusual case of anticardiolipin antibody related chorea in an adolecent male presenting to secondary care services.Case reportOur patient is a 14 year old male who became unwell in December 2016 with gradual onset of uncontrolled movements in his arms, and behavioural changes. There was a notable history of minor head injury, preceding sore throat and recurrent tonsillitis. Over the next six months, he reported worsening of choreoathetoid movements affecting his gait and upper limb function.He presented in April 2017 with acute slurring of speech three weeks after starting baclofen. He was then referred to tertiary paediatric neurology services. No features of Lupus were present on examination. His functioning was monitored after baclofen was stopped, and he progressed to treatment with high dose steroids, penicillin V and sodium valproate. He improved following significant rehabilitation with multidisciplinary involvement from occupational therapy, physiotherapy and the school team. His final diagnosis was revised to anticardiolipin antibody related chorea following results of immunological tests and consideration of the chronic progressive history. He was treated successfully with aspirin and mycophenolate mofetil, and has been discharged home with ongoing clinc review.Results12 lead ECG, echocardiogram and MRI brain normal.ASO Titre: 400 units/ml (50–200)Anti-DNAse B: 100 units/ml (<240 units/ml)Anti-Mitochondrial Ab: positiveAnti-Nuclear Ab titre: 1:320 (Positive)Anti-cardiolipin IgG Ab 1184.2 units/ml (0–19.9)Anti-B2-GP-1 IgG 4269.7 units/ml (<20)ConclusionAs a patient’s disease continues to evolve, so should our diagnostic approach. Atypical progession of disease should prompt review. Generally, treatment of anticardiolipin related chorea falls into 2 pathways; anticoagulation and immunosuppression, with evidence limited to case reports and small case series.Acknowledgements to the local medical illustration team for performing serial videos showing improvement in function.

Non-pharmacological rehabilitation interventions for individuals with antiphospholipid syndrome: A scoping review

“…Only four studies of non-pharmacological interventions with an APS-specific sample appeared in our search, and they contained only physiological outcomes (e.g., heart rate, time-to-exhaustion, VO2 peak) rather than patient-reported outcomes. 40–43 Of note, three of these four studies contained data from the same participants. 41–43 We did find one study that contained patient-reported outcomes 44 and described perceptions of barriers to engaging in physical activity and exercise from the perspective of individuals with APS, 26 but it did not test a specific intervention.

Figure 1.PRISMA flow diagram.

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“…The other APS-specific case study we identified reported involving rehabilitation professionals in the treatment of a 14-year-old male patient. 40 The treatment significantly improved his choreoathetoid movements in both upper and lower extremities, though no patient-reported outcome measures were listed. Overall, we found a dearth of evidence regarding non-pharmacological interventions and APS.…”